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4.4.5. Fontan Palliation of Single-Ventricle Physiology
COR LOE
Recommendations
Diagnostic
1 B-NR
1. In adults with Fontan circulation and evidence of progressive
Fontan circulatory failure, formal evaluation by a heart failure/
transplant cardiologist with experience in heart transplantation
in the adult Fontan population is recommended.
1 C-LD
2. In adults with Fontan circulation, imaging and laboratory
evaluation of the liver should be performed at least annually
to screen for hepatocellular carcinoma and for evidence of
progressive Fontan-associated liver disease (FALD).
1 C-EO
3. In adults being considered for Fontan palliation or Fontan
conversion, cardiac catheterization should be performed to
ensure the presence of acceptable hemodynamics.
1 C-EO
4. In adults with Fontan circulation and new-onset or worsening
atrial tachyarrhythmias, evaluation with imaging and follow-
up cardiac catheterization as indicated is recommended to
assess for potential contributory anatomic or hemodynamic
abnormalities and Fontan-pathway thrombosis.
1 C-EO
5. In adults with Fontan circulation and new or progressive
symptoms, hypoxemia, declining functional status, or
evidence of progressive or new-onset noncardiac organ
dysfunction, hemodynamic evaluation with cardiac
catheterization is recommended to guide therapy.
1 C-EO
6. In adults with Fontan circulation and newly progressive
or severe hypoxemia or hypotension, evaluation with
advanced cardiac imaging (MR, CT, transesophageal
echocardiography) is recommended to rule out thrombus or
emboli in the Fontan or pulmonary vasculature.
1 C-EO
7. In adults with Fontan circulation undergoing CT
angiography to rule out thrombus or emboli in the Fontan or
pulmonary vasculature, imaging should be performed using
protocols to avoid false-negative or false-positive results.
1 C-EO
8. In adults with Fontan circulation, annual laboratory evaluation
is recommended to assess for evidence of organ-system
dysfunction or hematologic abnormality.
2a C-EO
9. In adults with Fontan circulation, consultation by a
hepatologist, in collaboration with an ACHD cardiologist,
is reasonable to facilitate interpretation of hepatic testing,
diagnose and treat complications related to portal
hypertension, and participate in timing and management
decisions specific to organ transplantation.
2a C-LD
10. In adults with Fontan circulation, liver biopsy can be helpful
to delineate the degree of hepatic fibrosis or cirrhosis before
consideration of transplantation.
